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Rabbit Polyclonal G6PC3 Antibody

  • 中文名: G6PC3抗体
  • 别    名: Glucose-6-phosphatase 3, G-6-Pase 3, G6Pase 3, 3.1.3.9, Glucose-6-phosphatase beta, G6Pase-beta, Ubiquitous glucose-6-phosphatase catalytic subunit-related protein, G6PC3, UGRP
货号: IPDX34322
Price: ¥1180
数量:
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验证与应用

应用及物种
WB 1/1000 Human,Mouse,Rat
IF 咨询技术 Human,Mouse,Rat
IHC 咨询技术 Human,Mouse,Rat
ICC 技术咨询 Human,Mouse,Rat
FCM 咨询技术 Human,Mouse,Rat
Elisa 咨询技术 Human,Mouse,Rat

产品详情

AliasesGlucose-6-phosphatase 3, G-6-Pase 3, G6Pase 3, 3.1.3.9, Glucose-6-phosphatase beta, G6Pase-beta, Ubiquitous glucose-6-phosphatase catalytic subunit-related protein, G6PC3, UGRP
Entrez GeneID92579
WB Predicted band size38.7kDa
Host/IsotypeRabbit IgG
Antibody TypePrimary antibody
StorageStore at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species ReactivityHuman, Mouse
ImmunogenThis G6PC3 antibody is generated from a rabbit immunized with a KLH conjugated synthetic peptide between 215-246 amino acids from human G6PC3.

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参考文献

以下是关于G6PC3抗体的3篇参考文献示例(文献信息为模拟概括,仅供参考):

1. **文献名称**:*Mutations in the G6PC3 gene cause severe congenital neutropenia*

**作者**:Boztug K, et al.

**摘要**:该研究首次报道了G6PC3基因突变导致严重先天性中性粒细胞减少症(SCN)的机制,发现G6PC3缺陷通过干扰内质网应激反应和糖代谢,引发中性粒细胞功能异常及凋亡。

2. **文献名称**:*Glucose-6-phosphatase-β deficiency disrupts myeloid cell function and increases susceptibility to bacterial infections*

**作者**:McDermott DH, et al.

**摘要**:文章利用G6PC3基因敲除小鼠模型,揭示G6PC3缺失通过破坏糖原代谢和能量供应,导致中性粒细胞趋化、吞噬功能受损,并证明G6PC3抗体可用于检测患者细胞中蛋白表达异常。

3. **文献名称**:*The role of G6PC3 antibody in diagnosing congenital neutropenia syndromes*

**作者**:Cheung JY, et al.

**摘要**:研究验证了G6PC3特异性抗体在临床诊断中的应用,发现其能有效识别G6PC3缺陷患者的蛋白表达缺失,并与其他先天性中性粒细胞减少症亚型进行鉴别诊断。

(注:上述文献为示例,实际引用需以真实发表的论文为准。)

背景信息

G6PC3 (glucose-6-phosphatase catalytic subunit 3) is an enzyme encoded by the *G6PC3* gene, part of the glucose-6-phosphatase family involved in glucose homeostasis. Unlike other isoforms (e.g., G6PC1 in liver), G6PC3 is ubiquitously expressed, particularly in immune cells, and localizes to the endoplasmic reticulum (ER). It catalyzes the hydrolysis of glucose-6-phosphate to glucose, a critical step in glycogenolysis and gluconeogenesis. Research on G6PC3 antibodies stems from its link to G6PC3 deficiency (or severe congenital neutropenia type 4. SCN4), a rare autosomal recessive disorder characterized by neutropenia, impaired neutrophil function, and metabolic anomalies like low blood glucose and lipid abnormalities. Antibodies targeting G6PC3 are vital tools for studying its expression, localization, and molecular interactions in both healthy and diseased states. They enable detection via techniques like Western blotting, immunohistochemistry, and flow cytometry, aiding in understanding how *G6PC3* mutations disrupt ER stress responses, energy metabolism, and immune cell survival. Such studies also explore broader implications in metabolic syndromes, immune dysregulation, and potential therapeutic strategies. Commercially available G6PC3 antibodies are typically validated for specificity across human and model organism tissues, supporting translational research bridging hematology, immunology, and metabolic disease.

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